Clinical Study

Mitochondrial Sensorineural Hearing Loss: A Retrospective Study and a Description of Cochlear Implantation in a MELAS Patient

Table 1

Clinical and molecular features of patients analyzed in our study.

Clinical syndromeMolecular defectHearing lossNon hearing lossTotal

MIDDA3243G404
MELASA3243G111021
MERRFA8344G617
PEOSingle deletion (4)
Multiple deletions (20)
42024
MNGIETYMP mutations314

MIDD: Mitochondrial inherited ciabetes and deafness; MELAS: mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes; MERRF: myoclonic epilepsy with ragged red fibers; PEO: progressive external opthalmoplegia; MNGIE: mitochondrial neurogastrointestinal Encephalomyopathy.